Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, Harefield NHS Foundation Trust, Sydney Street, SW3 6NP London, UK.
National Heart and Lung Institute, Imperial College London, UK.
Eur Heart J Qual Care Clin Outcomes. 2018 Jan 1;4(1):51-58. doi: 10.1093/ehjqcco/qcx031.
The New York Heart Association functional classification (NYHA class) is often used to describe the functional capacity of adults with congenital heart disease (ACHD), albeit with limited evidence on its validity in this heterogeneous population. We aimed to validate the NYHA functional classification in ACHD by examining its relation to objective measures of limitation using cardiopulmonary exercise testing (CPET) and mortality.
This study included all ACHD patients who underwent a CPET between 2005 and 2015 at the Royal Brompton, in whom functional capacity was graded according to the NYHA classification. Congenital heart diagnoses were classified according to the Bethesda score. Time to all-cause mortality from CPET was recorded in all 2781 ACHD patients (mean age 33.8 ± 14.2 years) enrolled in the study. There was a strong relation between NYHA class and peak oxygen consumption (peak VO2), ventilation per unit in carbon dioxide production (VE/VCO2) slope and the Bethesda classification (P < 0.0001). Although a large number of 'asymptomatic' (NYHA class 1) patients did not achieve a 'normal' peak VO2, the NYHA class was a strong predictor of mortality, with an 8.7-fold increased mortality risk in class 3 compared with class 1 (hazard ratio 8.68, 95% confidence interval: 5.26-14.35, P < 0.0001).
Despite underestimating the degree of limitation in some ACHD patients, NYHA classification remains a valuable clinical tool. It correlates with objective measures of exercise and the severity of underlying cardiac disease, as well as mid- to long-term mortality and should, thus, be into incorporated the routine assessment and risk stratification of these patients.
纽约心脏协会功能分级（NYHA 分级）常用于描述成人先天性心脏病（ACHD）患者的功能能力，尽管其在这种异质性人群中的有效性证据有限。我们旨在通过检查 NYHA 功能分级与心肺运动测试（CPET）和死亡率相关的客观限制测量值来验证 ACHD 中的 NYHA 功能分级。
本研究纳入了 2005 年至 2015 年在皇家 Brompton 医院接受 CPET 的所有 ACHD 患者，根据 NYHA 分级对其功能能力进行分级。先天性心脏病诊断根据 Bethesda 评分进行分类。在研究中纳入的 2781 例 ACHD 患者中，所有患者均记录了从 CPET 开始至全因死亡的时间。NYHA 分级与峰值摄氧量（peak VO2）、二氧化碳产生单位通气量斜率（VE/VCO2）和 Bethesda 分类之间存在很强的相关性（P<0.0001）。尽管大量“无症状”（NYHA 分级 1）患者未达到“正常”峰值 VO2，但 NYHA 分级是死亡率的强有力预测因素，与 NYHA 分级 1 相比，NYHA 分级 3 的死亡率风险增加了 8.7 倍（危险比 8.68，95%置信区间：5.26-14.35，P<0.0001）。
尽管 NYHA 分级在某些 ACHD 患者中低估了限制程度，但它仍然是一种有价值的临床工具。它与运动的客观限制测量值和潜在心脏疾病的严重程度相关，以及中至长期死亡率相关，因此应纳入这些患者的常规评估和风险分层。
